...Roman Szabo; Makiko Kawaguchi; Hiroaki Kataoka; Thomas H. Bugge ABSTRACT Congenital tufting enteropathy (CTE) is a life-threatening intestinal disorder resulting from loss-of-function mutations in EPCAM and SPINT2 . Mice deficient in Spint2 , encoding the protease inhibitor HAI-2, develop CTE-like...

...-associated loss of the cell junctional proteins EpCAM and claudin 7 was also prevented. As a result, inactivation of intestinal matriptase allowed Spint2 -deficient mice to gain weight after birth and dramatically increased their lifespan. These data implicate matriptase as a causative agent...

... and in the in vitro colony formation assay. We demonstrate that epithelial cell adhesion molecule (EpCAM) is expressed in both mouse normal cholangiocytes and oval cells, whereas its related protein, TROP2, is expressed exclusively in oval cells, establishing TROP2 as a novel marker to distinguish oval cells from...